22-34: Nephrogenic Systemic Fibrosis

Nephrogenic systemic fibrosis is a multisystem disorder seen only in patients with CKD (primarily with an eGFR less than 15 mL/min/1.73 m², but rarely 15–29 mL/min/1.73 m²), AKI, and after kidney transplantation. Histopathologically, there is an increase in dermal spindle cells positive for CD34 and procollagen I. Collagen bundles with mucin and elastic fibers are also noted.

Nephrogenic systemic fibrosis was first recognized in hemodialysis patients in 1997 and has been strongly linked to use of contrast agents containing gadolinium. Incidence following gadolinium injection is approximately 1–4% in the highest risk (ESKD) population and lower in patients with less severe kidney dysfunction. The incidence has decreased over time due to limiting use of gadolinium in patients with CKD and AKI and modified gadolinium preparations. There is an FDA warning regarding avoidance of this agent for patients with an eGFR less than 30 mL/min/1.73 m².

CLINICAL FINDINGS

Nephrogenic systemic fibrosis affects several organ systems, including the skin, muscles, lungs, and cardiovascular system. The most common manifestation is a debilitating fibrosing skin disorder that can range from skin-colored to erythematous papules, which coalesce to brawny patches. The skin can be thick and woody in areas and is painful out of proportion to findings on examination.

TREATMENT

Case reports and series describe benefit of corticosteroids, photopheresis, plasmapheresis, and sodium thiosulfate, but their true efficacy is unknown. CT is preferred to MR imaging with gadolinium when similar diagnostic information can be gleaned. If gadolinium absolutely must be used in patients on dialysis, practice guidelines recommend using no more than the standard dose and hemodialysis immediately after exposure.